Tuesday, May 29, 2012

"This disease must do wonders for my appearance because everyone keeps telling me how good I look!"

I saw this on a t-shirt and I thought "so true!". Everyone's always comments on how good Drew looks. And while I do appreciate it, it sometimes annoys me. What do you expect, a third arm? Like they're thinking "You harp on how sick he is and what a horrible disease this is and then he looks "normal" so he must be fine." I was told from the beginning that the blessing and the curse of CF was that there were very few outward signs of the disease. The blessing being that most people would treat him just like anyone else. The curse that people never gauge the full impact of the disease, or what we do to keep him looking the way he does. We spend hours, literally hours, every day doing breathing treatments and airway clearance. We give him up to 25 pills a day, in addition to 8 other medicines. Nutrition is equally as important because his pancreas is broken so his body doesn't absorb fat. And he dehydrates super easily so he needs a lot to drink, especially in the summer when he is outside and sweating. I hate making CF the topic of every get together, but I can't help but explain after I announce that he can't have his fruit until he eats all of his chicken nuggets or why I'm giving a 2yr old Gatorade. It's the opposite of what you do with a non-CFer. But he needs the fat and the sodium.

At our last clinic visit, his weight was down ever so slightly. We have never had a problem with his weight. It's actually been surprisingly good from the get go. Our clinic aims to get kids into the 50 percentile for weight and he's hanging somewhere around 80-85th and has been since before her turned 1. But at the last visit they noticed a small dip and told us to keep an eye on it because a dip can start a trend and we cant afford that. So we've been getting monthly weight checks at our pediatrician and he was going up about a quarter of a pound a month, until today when he was down a pound and change. The pediatrician commented that his weight was not at all concerning to her, he's still in the 80th percentile. It's not his weight that concerns me, it's his weight loss. Admittedly he has been sick and on antibiotics, but I still want to be able to at least maintain.

Drew is a terribly picky eater. Or a control freak. I don't know which. He won't eat much of anything, which is surprising based on his size. I want to treat him like the girls and offer him what we are having for dinner and if hes hungry then he will eat it and if not then he will just have to deal. And I do do that sometimes, but I find myself pouring him a Ensure Plus just to get some calories in. There have been days where he will have absolutely nothing but milk and he doesn't seem phased by it whatsoever. I, on the other hand, worry like a fool. I don't want him thinking that's okay. But maybe he's just not hungry. Or maybe it's the one thing that he can control in his crazy world of treatments and medicines and he knows it gets my attention so he plays that card because he's 2 and he can. I just don't know. We saw a food psychologist a little while ago who said to try to ignore him when he doesn't eat, but when he does we should respond with over the to praise - yelling, clapping, etc. We try that from time to time and sometimes it works but most of the time if he doesn't want to eat, he's not gonna eat. Tonight I told the kids they could have a special treat if they ate their dinner. The girls ate and got ice cream. He didn't eat but so badly wanted the ice cream and I so badly wanted to give it to him to get something in him, but I had to stick to the rules I set. Its better for our family in the long run. When the girls went away to play I gave him 4 scoops of Nutella, probably equal to at least as many calories as the hamburger I tried to get him to eat, if not more.

They recommend cutting his milk with heavy cream and putting butter on toast under the peanut butter. We search the grocery store for high fat yogurt, which is a chore in a world where everyone wants low fat everything. We add oil or butter and cream and as many toppings as we can to whatever he eats to pack a little extra in but I still worry. I know every kid, even every CF kid has difference caloric needs. Thank God that he holds on to the little fat that he does get or we would have a bean pole on our hands. Unfortunately, a lot of kids entering the late toddler to early adolescent years end up getting a G-Tube to feed them intravenously overnight and thought the day to help get all of the calories that they need to thrive. And while with CF we simply do what we have to do, I don't want a G-tube. I don't want another surgery. What I really want is to try Kalydeco and see if it helps Drew, but unfortunately we're just not able to yet. Anyone who has secret ways of getting in extra calories for their CFer, please share.

On another note altogether, we found this super great neb mask holder that we absolutely love. Drew uses the fish masks and we were having some trouble getting the strap to stay put and not slide down. Enter Strap & Snap. This mask stays put and is comfortable and cute. I highly recommend it to anyone using a neb mask!

Finally, we had our second annual CF Yardsale this past weekend and raised $500! Yay for people who like to buy junk! And this concludes our fundraising for a few months until our Oktoberfest party in late September (save the date!). I'll definitely still be posting about different CF events too, just in case anyone wants to participate in anything!

Tuesday, May 22, 2012

Treatments have gotten harder lately. Not harder in that Drew doesn't want to do them, but harder in that Drew doesn't want to do them without sitting on my lap. And with 3 other kids needing my attention it's just gotten hard. He's always sat with someone to do them, but usually in the morning either Martin is here to help with the others or I can do them after I drop Ella off at school while Jake is sleeping, and in the evening Martin is home as well. Now, between school being out and Drew being sick and Jake seemingly wanting to eat ever 35 seconds, I feel like I'm glued to the couch and I can recite every line to Toy Story (1, 2 and 3). And I feel like Drew deserves my attention while he's sitting here, 3 times a day for 45min. He didn't ask for this and certainly doesn't want to stop playing with his trucks to do it as often as he had to. But the girls get the short end of the stick too, being told to wait all the time or getting in trouble for misbehavior that happens while I'm not watching. It's not really fair to them either. I sometimes feel like the only "quality time" Drew gets is while he's doing his treatments, and by "quality time" I mean time sitting on my lap watching TV - decidedly not QT. However, trying to squeeze in that time to play with each kid, individually or just aside from the rest of the group is tough. I make a lot of promises for later that are rarely followed through on because someone always needs a diaper change or a drink refill or a boo boo kissed. And I feel bad about it all. There's a part of me that wants to spend some time for me too, but it always gets put below something else because there never seems to be enough time. This is what I hate about CF.

I'm going to try this summer to make the most of the time we have to play. The phone will be put down and I'll try my hardest to plan ahead so as to be ready to, for example, go to the park when the baby is fed and diapers are changed and treatments are done. I know everyone around here is happier when they just get a little attention. I like to (and need to sometimes) sit down and drink a cup of coffee and do nothing for an hour, but the days where I take a 5 minute break I end up 5 minutes behind schedule and things get missed and life begins to snowball.

I don't mean to complain. I sometimes just wish this was all a little easier. I notoriously over extend myself with extracurricular activities, but it's all with good intent. Hopefully as we get a little deeper into our summer groove I'll start feeling better about everything. Of course by that time school will be ready to start again!

Monday, May 21, 2012

Our walk was this weekend and it was a huge success! We (Cincinnati, not our team) raised $340,000. The weather was perfect and the new route along the river was great. We have some extra shirts this year for anyone who wants to buy them. We are selling them for $5 + shipping (so pretty much $10 unless you live in another country). We also had our walks in Chicago, Buffalo, New London and Charleston. Still to come are Hawaii and New York. We're so happy that we have such great family and friends walking for Drew all over the country. Here are a couple of my favo pix from our big day:

Drew is still sick. We got put on Amoxicillin last week but he's still coughing a ton. I talked to his doctor again today and we switched antibiotics. He usually has a pretty good response to Omnicef so hopefully in another day he sounds at least improved if not totally back to normal. This illness just makes me want to get on Kalydeco more. It may not help him at all, but I'll never know unless I try it. If only it was available to me (sigh). Soon enough I suppose...but not soon enough.

Summer has officially begun here. School is out, dance is done and we are playing in the sprinkler and/or pool almost daily. The weather has been gorgeous but its supposed to get up to 90 here this weekend which will move us back indoors because of Drew's inability to tolerate such heat and humidity. He has so much trouble breathing when its so hot and humid here. We put a window AC unit in his bedroom so that its extra cool and comfortable for him to sleep.

I wish I had more time to blog. I have great thoughts I'd love to put down here, but they leave me as quickly as they come to me and I just can't seem to find the time to open the computer anymore. If only there were a few more hours in a day.

Thursday, May 17, 2012

I am exhausted. I don't know what happened this month. I thought we were going to have an easy transition into summer, with the end of school, dance and gymnastics all this week. But between shows and appointments and kids getting sick, I am completely beat.

Drew is sick. He's had a lingering "cold" for about 3 weeks now and it wasn't really getting any better or worse so I was just kind of keeping an eye on things. He had a follow up appointment at his ENT last Friday and they said the nasal polyps he had scraped out back in Feb had grown back. That's such a bummer. The doctor reiterated that he had pretty aggressive sinus disease and would probably need surgery again sooner rather than later to get that under control. We knew it would happen again but were hoping for more like a year, not 6mo. It wasn't the worst surgery he's ever had, but having to get put under for anything is no fun, and he was in some serious pain when he came out of it. I guess we will wait and see.

While we were there, I asked them to take a culture to see if there was any bacteria growing that we needed to treat or if the "cold" was just a viral thing that was going to go away on its own. (For what its worth, he's never gotten over a virus on his own. It always turns into a bacterial infection. Maybe someday.) He grew haemophilus influenza (h-flu). Its a pretty common bacteria, something that causes ear infections or sinus infections and not at all specific to CF. Regardless, he grew it and we decided to treat. And I'm glad that we did because overnight he went from fine to terrible. He was coughing up a storm all day yesterday and not interested in eating or drinking. He just wanted to be held and put his head on my shoulder and I obliged as much as I could but there are 3 others also needing my attention so it was a rough day. When he's sick, our already packed days get even more full, with increased breathing treatments and airway clearance. I estimate that yesterday, between breathing treatments and time spent nursing the baby, I spent between 4-5 hours sitting on the recliner with a kid in my lap. That left many children bouncing off the wall and acting up because they weren't getting my attention. To top it all off, last night was Ella's dance recital and I had agreed to be the stage mom which meant arriving an hour early. I know it sounds crazy that I would volunteer to do something extra, but I so rarely have time to give my girl undivided attention and the twins were staying home so I thought she would like it. With the way our week was going, I regret my commitment, but we made it and she danced and looked adorable and I took pictures...and now we are done with dance.

I found a new website this week called "But You Don't Look Sick". It written for/about living life to the fullest with any disability, invisible disease or chronic pain. The woman who writes the blog said the following:"The difference in being sick and being healthy is having to make choices or to consciously think about things when the rest of the world doesn't have to. The healthy have the luxury of a life without choices, a gift most people take for granted." Gosh, if that wasn't my week to a T. Or my life really. And I hate it. I wish we could just go with the flow some days, and I certainly try my darnedest to make it seem like we do. But CF forces us to make choices. Choices about everything from treatments and medications to play dates with friends and simple family activities. When Drew is sick and we have to squeeze in extra stuff, it means we don't get to go to preschool end of the year parties, which disappoints someone. So we decide to try to do a small park ourselves which is closer. But we have to wait until we get his breathing treatments in. Then the baby needs to be nursed. And someone (me) needs to pack the diaper bag and make sure everything from drinks to inhalers are in the bag. And then we need to make sure everyone who uses the potty has gone because there is no bathroom at our small, manageable park. And now, at best, we will only have an hour at the park because we need to get home for lunch and naps because we have a doctor appointment in the afternoon to get to. And the baby needs to be nursed before we go. And I forgot to buy tights for the dance recital, but can't figure out when I will have a free minute to get to Target to get them. And oh yeah, the antibiotic that the doctor called in needs to be picked up from the pharmacy too. And this is all just the stuff that has to happen. Its not the extra stuff. Its not the dinners with friends and walks in the evening or the 20 minutes of free time to sit down and blog it all out. I hate having to think about every little detail all of the time. I wanna decide, while I'm out with friends, to have an extra drink and come home a little late. But when I do that, I'm extra tired and the nebs aren't cleaned for the morning because I get home late and just want to go to bed. But I have to pump first. Ugh. And I think often times people don't understand or get offended by my inability to commit to the non-essentials because they don't have to think about all of this. I don't like having to make these choices, but its my job. Even this weekend, our walk is on Saturday at 10am. And i'm already planning how we will have to get everything together and ready the night before, because if we wanna be there by 9:30 we will have to get up by 7 at the latest to get in all the treatments, feed everyone, sunscreen up, etc, etc, etc. And I can't forget the bonus insurance phone calls I get to deal with. Last week they totaled about 5 hours. And I'm not even exaggerating in the least; check my phone records. Sorry about the rant. Its just how things have been lately, and when I read that line from the new blog that I found, it hit home.

Now its 11pm and I already regret staying up this late. We have nothing going on tomorrow, or pretty much for the rest of June, and come July we are heading east, never to return. Ok. that's not even kind of true. But in my imagination, I'm going to leave all of our worries behind and visit with our family and friends up and down the east coast without a care in the world. The reality is that I'll probably spend half of the trip on the phone with the doctor or insurance company trying to explain why and how we need medication shipped to a pharmacy in Pennsylvania. Sigh. While today was filled with medications and an unhappy little boy, at least we didn't have to schedule around appointments and other commitments.

Wish us luck at our walk! We didn't hit our number for this year, but I did the best I could. There's still time to donate! Money buys science and science saves lives. And once that cure is found, I imagine my schedule opening up a bit :)

Tuesday, May 8, 2012

The Goat Races were this weekend. Unfortunately Curly didn't win the whole thing, but he did win the first heat he ran in. My parents collected $280 in random donations for the CFF at the event, and hopefully were able to raise a fair amount of awareness for CF.

Big news this week for the CF world! An email popped up in my inbox titled "Phase 2 Study of Kalydeco and VX-809 in Combination Shows Promising Interim Results". Hooray for VX-809! This is the drug that works on the DeltaF508 mutation. It was shown to improve lung function as much as 10% in some patients! This is the kind of drug that is going to cure CF.

In discussions I've had with some friends recently, I think people have a hard time sometimes understanding exactly what the problem is with CF in relatively simple terms. I found this on Johns Hopkins CF website and thought it explains it pretty well. Its a little lengthy but read it through:

Sweat is produced in glands under the skin, transported to the skin surface through ducts, where some of the salt and water are reabsorbed. In normal skin functioning, as sweat moves along the duct most of the sodium and chloride -- the components of salt -- are reabsorbed. This is driven by a large force drawing sodium into cells lining the sweat duct. The chloride flows through the CFTR channel, while sodium flows into the cell through epithelial sodium (Na) channels (ENaC) in the apical membrane.

The salt reabsorption process is markedly abnormal in people with CF. Chloride transport is virtually eliminated because CFTR, which is the main way for chloride to enter the apical, or top, surface of cells is defective. Chloride must travel through the CFTR, located on the surface of the duct cells, at the same rate as sodium to maintain a balance of electrical charges. This results in very little sodium and chloride reabsorption, leading to a high salt content in CF sweat -- so high that the sweat chloride concentration is the most reliable single test for CF.

The lung’s airways are covered with a thin, moist film called airway surface liquid, or ASL, a salt-containing liquid and a mucus gel layer. The ASL traps bacteria and foreign particles, while cilia on the surface of airway cells constantly move the particles out of the lungs and toward the mouth. This process, called mucociliary clearance, or MCC, is an important defense mechanism that protects the lungs from infection. ASL also contains antiproteases, antioxidants, antibodies and other substances that work together to neutralize or destroy invading organisms without damaging the lungs. In CF airways, decreased chloride transport is coupled with excess sodium transport out of the surface liquid. Since water follows the flow of sodium, the ASL loses volume and the mucus gel layer becomes dehydrated.

There are thousands of different mutations of genes that can cause CF. Drew has one called ΔF508 and another called R560T. Mutations are grouped into classes based on the exact problem with the mutated cells. Click here for a little bit about the problems the different mutations cause. ΔF508 is a class 2 mutation. I, having no clue how cells work, rely on the analogies given by certain medical professionals to understand things. They often refer to the processing problem of a CF cell as a locked door and the sodium chloride either can get to the door but cannot open the door (class 3 problem) to get to the right place or it not being able to find the door in the first place (class 2 problem). You may recall several months ago the HUGE CF world news that a drug was developed that corrects the problem of class 3 mutations. When people take this drug, the problem of the broken CFTR gene not being able to open the door when it gets to the door is corrected. The data that was just released yesterday announcing promising results for the combo drug study uses that drug that came out in January in combination with a drug that leads the broken CFTR gene to the door, thus Class 2 mutations can both find and then open the door to get to the right place. I know its all a little confusing. If you EVER have the chance to hear Dr. JP Clancy speak anywhere, go. He is a brilliant man, and he is amazing and dumbing it down to a non-medical level (the door analogy). Its really pretty cool stuff, all thats happening in the CF world right now. I have so much hope for a different future!

Thursday, May 3, 2012

I know this is kind of late notice, but tonight on the Opran Winfrey Network at 9 pm EST there is a documentary about Cystic Fibrosis. It got rave reviews by the NY Times and is chronicles the life one woman waiting for a lung transplant. If you don't know a lot about CF, this should add some real perspective about the realities of the disease. Watch it!

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org